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PubMed2004

Human leukocyte antigen class II associations with systemic sclerosis in South Africans.

Human leukocyte antigen class II typing, using polymerase chain reaction-sequence-specific primers, was performed in 52 Black South Africans with systemic sclerosis (SSc) and 112 controls. Increased frequencies of DR2 in the overall SSc group (OR = 2.4), DRB1*0301 in the limited cutaneous SSc (lcSSc) subset (OR = 9.0), and DQB1*0301/4 in the diffuse cutaneous SSc (dcSSc) subset (OR = 9.0) were observed. Pulmonary fibrosis was associated with DRB1*11 and anti-topoisomerase I antibodies were associated with DPB1*1301 and DRB1*15. Patients with anti-fibrillarin antibodies (AFAs) had increased the frequencies of DRB1*1101 allele group (OR = 16) and DQB1*0603/14 (OR = 13.6). These findings provide new serological and immunogenetic data on a previously unreported population. The association of AFAs with class II alleles merits further investigation.

Africa, Southern↗
PubMed1975

[Systematic study of sequential salivary gland scintigraphy during rheumatoid arthritis and collagenosis. Apropos of 150 examinations].

Sequential salivary scintigraphy has been practised systematically in 130 patients hospitalized in a rheumatology unit. These patients were suffering from typical, classical rheumatoid arthritis, rheumatoid arthritis with high positivity for anti-nuclear antibodies, clearly defined polyarthritis, systemic scleroderma, other types of collagenosis, forms of inflammatory polyarticular rheumatism other than rheumatoid arthritis and collagenosis, and from different degenerative diseases. The scintigraphic anomalies were divided in three stages according to a personal classification. Nine patients presented a patend Gougerot-Sjögren syndrome. All of them had abnormal or very abnormal scintigraphy. However, the patients with classical rheumatoid arthritis without signs of the Gougerot-Sjögren syndrome, also showed a high frequency of isotopic anomalies that were equally severe. These were clearly connected only with the particular diagnosis (with classical rheumatoid arthritis, but not with clearly defined polyarthritis or collagenosis). No other direct relationship could be established between these diseases and the clinical, radiological, laboratory, or therapeutic aspects of the case. On the other hand, they were often associated with a reduction in lacrymal and salivary secretion. These isotopic studies, in combination with other findings shed a new light on the Gougerot-Sjögren syndrome, taking into account the diffusion of the exocrine lesions and of the contributions of the most modern methods of investigation.

Antibodies, Antinuclear↗
PubMedDate not supplied

Redistribution of pulmonary blood flow in interstitial lung diseases: the chest radiograph as a physiologic tool.

Posteroanterior radiographs of the chest showed enlargement of vessels in the upper lung fields in 18 of 29 patients with interstitial lung diseases, despite normal pulmonary wedge pressures and normal or reduced pulmonary blood volumes. The degree of such redistribution ("diversion") did not correlate either with the severity of pulmonary hypertension observed at cardiac catheterization or with radiologic assessment of predominance of disease at the lung bases. Diversion did correlate with several indices of disease severity: reduction in vital capacity, reduction in diffusing capacity, reduction in pulmonary blood volume and radiographic severity of parenchymal abnormalities. Furthermore, diversion correlated with lung height, a variable which was not statistically related to the other indices of disease severity. Distension of upper lung vessels occurs in interstitial lung diseases as the result of a decreased hydrostatic gradient over which the lung is perfused (decreased lung height), partial obliteration of the vascular bed (decreased pulmonary volume), and, more speculatively, decreased extravascular pressure (increased lung recoil).

Arthritis, Rheumatoid↗
PubMed2004

CT features of lung disease in patients with systemic sclerosis: comparison with idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia.

PURPOSE: To evaluate computed tomographic (CT) patterns of lung disease in patients with systemic sclerosis (SSc) and compare them with CT appearance in patients with biopsy-proved idiopathic pulmonary fibrosis (IPF) and idiopathic nonspecific interstitial pneumonia (NSIP). MATERIALS AND METHODS: The CT features of consecutive patients with SSc (n = 225; male patients, 44; female patients, 181; median age, 47 years; age range, 16-78 years), IPF (n = 40; men, 26; women, 14; median age, 54.5 years; age range, 36-77 years) and NSIP (n = 27; men, 18; women, nine; median age, 53 years; age range, 32-68 years) were quantified separately by two observers. The extent of interstitial lung disease, ground-glass opacification, emphysema, and the coarseness of a reticular pattern were quantified. Group comparisons were made nonparametrically with the Wilcoxon rank sum test. Differences in CT features were identified with multiple logistic regression analysis. RESULTS: The coarseness of fibrosis was similar in patients with SSc and idiopathic NSIP but strikingly different between patients with SSc (median coarseness score, 5.5; range, 0.0-13.3) and IPF (median coarseness score, 8.8; range, 2.5-15.0) (P <.001). The proportion of ground-glass opacification at CT was similar in patients with SSc and idiopathic NSIP but differed significantly between patients with SSc (median proportion, 49.9%; range, 0.0%-100.0%) and IPF (median proportion, 23.5%; range, 0.0%-97.2%) (P <.001). At logistic regression analysis, there were no differences in the CT features between patients with SSc and those with NSIP after controlling for age, disease extent, and the percentage predicted forced vital capacity and carbon monoxide diffusing capacity. CONCLUSION: Interstitial lung disease in patients with SSc is less extensive, less coarse, and characterized by a greater proportion of ground-glass opacification than that in patients with IPF. The CT features of lung disease in patients with SSc closely resemble those in patients with idiopathic NSIP.

Adolescent↗
PubMed1993

Pulmonary involvement in progressive systemic sclerosis: sequential evaluation with CT, pulmonary function tests, and bronchoalveolar lavage.

Computed tomographic (CT) findings obtained in 53 patients with progressive systemic sclerosis were correlated with functional parameters and bronchoalveolar lavage (BAL) results, and lung changes over time were assessed in 17 patients. CT findings were normal in 21 patients (group 1) with otherwise normal lung function, except for subclinical alveolitis in seven patients. CT depicted pleural and parenchymal abnormalities in 32 patients, grouped according to the absence (group 2) or presence (group 3) of honeycombing. In group 2 (n = 13), mean values of functional parameters were normal, and BAL showed a significant increase in neutrophils compared to group 1 (P < .05). Among patients in group 3 (n = 19) with limited extent of honeycombing (n = 12), the mean diffusing capacity value was lower in patients with a moderate ground-glass profusion score (n = 4) than in those with a mild score (n = 8) (68% +/- 4 [standard error of the mean] vs 80% +/- 3). CT is the method of choice for evaluating parenchymal destruction, and profusion and extent of ground-glass opacities can help in predicting the severity of lung damage in areas devoid of destructive changes.

Adolescent↗
PubMed2003

Analysis of lymphocyte subpopulations in systemic sclerosis.

Systemic sclerosis (SSc) is a chronic inflammatory connective-tissue disease of unknown etiology, characterized by fibrosis and microvascular injury in affected organs. It has become clear that the activated cellular-immune system plays a central role in the pathogenesis of SSc. This study analyzes the numbers of lymphocyte subpopulations and their relations with clinical and laboratory manifestations. We studied a group of 42 patients with SSc and a group of 28 matched normal controls by flow cytometry using the lymphocyte cell-surface markers CD2, CD3, CD4, CD8, CD19, CD25, CD45RA, CD56, CD71, HLA-DR, TCR alpha/beta, and TCR gamma/delta. Patients with SSc had similar percentages of CD2+, CD3+, CD3+ CD4+, CD3+, CD8+, CD25+, CD4+ CD45RA+, CD8+ CD45RA+, CD71+ cells, and CD4+/CD8+ cell ratio when compared to normal controls. In contrast, the percentages of TCR gamma/delta cells were significantly lower in SSc patients with diffuse and late-stage disease with pulmonary involvement, muscle involvement, and the presence of anti-Scl-70 antibodies. Patients with diffuse SSc in early- and late-stage disease had significantly increased percentages of HLA-DR in CD4+ and CD8+ cells. Patients with late-stage disease had increased percentages of CD4+ CD45RA+ T-cells. Patients with limited and early-stage disease had smaller percentages of B-cells (CD19+). Patients with diffuse and late-stage disease had smaller percentages of NK-cells (CD56+). These results suggest that T-, B-, and NK-cell alterations may be involved in the onset of the disease, and/or in the perpetuation of disease, and may eventually be useful as a prognostic indicator in selected patient subgroups.

Adult↗
PubMed1995

Increased susceptibility to oxidation of low-density lipoproteins isolated from patients with systemic sclerosis.

OBJECTIVE: To examine the resistance to oxidation of low-density lipoproteins (LDL) from patients with systemic sclerosis (SSc) and primary Raynaud's phenomenon (RP) compared with healthy controls. METHODS: Plasma LDL were isolated from patients with diffuse cutaneous and limited cutaneous SSc (dcSSc and lcSSc, respectively), patients with primary RP, and healthy control subjects. The lipoproteins were assessed for their resistance to oxidation in the presence of cupric ions, using spectrophotometric assays. RESULTS: LDL from patients with dcSSc and lcSSc were more susceptible to oxidation than were those from healthy control subjects or patients with RP. CONCLUSION: Our findings suggest that free radicals may play a role in the pathology of SSc.

Aged↗
PubMed2004

The diagnostic value of oesophageal transit scintigraphy for evaluating the severity of oesophageal complications in systemic sclerosis.

BACKGROUND: Oesophageal complications are common in systemic sclerosis (SSc). However, the ability to determine the severity of oesophageal complications according to SSc type and skin lesion has not been evaluated. METHODS: The study groups consisted of 35 patients with SSc who were classified into diffuse (n=20) and limited (n=15) cutaneous types, and 16 control subjects. An additional 26 consecutive patients were studied for an analysis of the reproducibility. The severity of a skin lesion was quantified by using a modification of Rodnan's total skin thickness scores. Oesophageal scans were performed after the subjects, in sitting and supine positions, had consumed potage soup. Condensed images of the dynamic study were classified into four patterns: normal, transient retention, slight retention and severe retention, in conjunction with parameters of retention fraction by analysing the time-activity curve. RESULTS: The highest reproducibility was obtained using retention at 90 s (R90, r=0.93). Analysis of the condensed images showed that the SSc patients had a higher incidence of severe retention than did the control subjects. Groups with diffuse-type SSc or a high skin thickness score showed a higher incidence of severe retention (P=0.041 and 0.0048, respectively) compared with the control and less severe groups. The R90 in the supine position differed significantly among the controls, the limited-type and diffuse-type SSc groups (mean+/-SEM, 10+/-1%, 24+/-5%, 38+/-6%, respectively; P=0.0004)). The group with high skin scores (i.e. > or =10) showed a higher R90 (41+/-6%) than did either the group with low skin scores (R90=23+/-5%) or the control group (P=0.0001). CONCLUSION: An oesophageal scan can detect both slight and severe types of oesophageal dysfunction, and can be used as a quantitative method that reflects functional abnormality in SSc.

Adult↗
PubMedDate not supplied

[Subclinical interstitial lung involvement in rheumatic diseases. Correlation of high resolution computerized tomography and functional and cytologic findings].

INTRODUCTION: Rheumatic diseases are frequently associated with interstitial lung disease. Since interstitial fibrosis is an irreversible process, understanding the mechanisms leading to fibrosis is necessary for the development of treatment strategies to prevent irreversible pulmonary damage. High-resolution Computed Tomography (HRCT) is superior to chest radiography in assessing the presence and extent of parenchymal abnormalities in diffuse infiltrative lung diseases and provides a sensitive and noninvasive method of quantifying global disease extent. PURPOSE: The aims of this study were to quantify the severity and extent of subclinical interstitial lung disease as depicted on HRCT and to study the relationship between the patterns of lung disease quantified by HRCT and the functional parameters and bronchoalveolar lavage findings in patients with rheumatic diseases. PATIENTS: Eighty nonsmoking patients (24 patients with systemic sclerosis, 24 with primary Sjögren's syndrome, 20 with rheumatoid arthritis and 7 with dermatopolymyositis) were examined. No patient had any signs or symptoms of pulmonary disease. RESULTS: Thirty-three of 80 patients (41.2%) had abnormal HRCT findings, namely isolated septal/subpleural lines, irregular pleural margins and ground-glass appearance. Chest X-ray showed parenchymal abnormalities in only 15 patients (18.7%) who had evidence of fibrosis on HRCT. Abnormal differential cell counts (alveolitis) at bronchoalveolar lavage were found in 46 of 80 patients (57.5%). Three types of alveolitis were observed: pure lymphocyte alveolitis, pure neutrophil alveolitis, and neutrophil alveolitis associated with lymphocytosis (mixed alveolitis). The patients with neutrophil alveolitis had more extensive disease on HRCT than those with lymphocyte alveolitis or with normal cellular patterns at bronchoalveolar lavage. The extent of a reticular pattern on HRCT correlated with the neutrophil rate (p = 0.001) and total count (p = 0.003) on bronchoalveolar lavage. Eosinophil and lymphocyte rate and total count correlated (p < 0.05) with the extent of the ground-glass pattern on HRCT. Lung volumes were not significantly different among patients with ground-glass pattern and those with reticular patterns on HRCT, while the diffusing capacity for carbon monoxide was significantly lower (p < 0.05) in the latter. CONCLUSIONS: HRCT is a sensitive tool in detecting interstitial lung disease in patients with rheumatic diseases with no signs and symptoms of pulmonary involvement. The relationship between the different HRCT patterns and bronchoalveolar lavage cell profiles can identify patients at higher risk of developing irreversible lung fibrosis. A long-term, prospective follow-up study is needed to determine whether these patients will develop over pulmonary disease.

Adult↗
PubMedDate not supplied

Bowed fingers. A helpful sign in the early diagnosis of systemic sclerosis.

Thirteen patients who had in common the clinical finding of shortened forearm flexors were investigated. Additional findings were Raynaud's phenomenon, arthralgia, reduced pulmonary CO diffusion capacity, abnormal EMG, and histological evidence of myopathy. Clinical and serological features in 2 patients were consistent with features described in the mixed connective tissue disease syndrome. The remainder were regarded as having a limited connective tissue disease syndrome, in all probability, a minimal expression of systemic sclerosis. We suggest that the finding of bowed fingers is a more reliable clinical sign than the assessment of skin texture in determining the presence of minimal connective tissue disease.

Adult↗
PubMedDate not supplied

Medical use of dimethyl sulfoxide (DMSO).

DMSO is a clear odorless liquid, inexpensively produced as a by-product of the paper industry. It is widely available in the USA as a solvent but its medical use is currently restricted by the FDA to the palliative treatment of interstitial cystitis and to certain experimental applications. Cutaneous manifestations of scleroderma appear to resolve (albeit equivocally) following topical applications of high concentrations of DMSO. A limited number of small clinical trials indicate that intravenous DMSO may be of benefit in the treatment of amyloidosis, possibly by mobilizing amyloid deposits out of tissues into urine. Dermal application of DMSO seems to provide rapid, temporary, relief of pain in patients with arthritis and connective tissue injuries. However, claims for antiinflammatory effects or acceleration of healing are currently unwarranted. There is no evidence that DMSO can alter progression of degenerative joint disease, and, for this reason, DMSO may be considered for palliative treatment only and not to the exclusion of standard antiinflammatory agents. The safety of DMSO in combination with other drugs has not been established; neurotoxic interactions with sulindac have been reported. In experimental animals, intravenous DMSO is as effective as mannitol and dexamethasone in reversing cerebral edema and intracranial hypertension. An initial clinical trial in 11 patients tends to support this latter application. DMSO enhances diffusion of other chemicals through the skin, and, for this reason, mixtures of idoxuridine and DMSO are used for topical treatment of herpes zoster in the UK. Adverse reactions to DMSO are common, but are usually minor and related to the concentration of DMSO in the medication solution. Consequently, the most frequent side effects, such as skin rash and pruritus after dermal application, intravascular hemolysis after intravenous infusion and gastrointestinal discomfort after oral administration, can be avoided in large part by employing more dilute solutions. Most clinical trials of DMSO have not incorporated the components of experimental design necessary for objective, statistical evaluation of efficacy. Randomized comparisons between DMSO, placebo and known active treatments were rarely completed. Final approval of topical DMSO for treatment of rheumatic diseases in particular will require a multi-center, randomized comparison between high and low concentrations of DMSO and an orally-active, nonsteroidal antiinflammatory agent.(ABSTRACT TRUNCATED AT 400 WORDS)

Animals↗
PubMed1985

Relationship between nailfold capillary abnormalities and organ involvement in systemic sclerosis.

Nailfold capillary abnormalities in 42 consecutive patients with systemic sclerosis were studied by wide field capillary microscopy, and capillary abnormalities were correlated with organ involvement. Twenty-eight patients hd diffuse skin disease, and 14 had the CREST variant of systemic sclerosis (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasis) with anticentromere antibodies. Nailfold capillary enlargement and loss were graded from photographs. There was no correlation between the severity of either nailfold capillary loss or enlargement and duration of disease, number of organ systems involved, or acroosteolysis. The presence of telangiectasis correlated with extreme capillary enlargement (P less than 0.025). Based on these findings it can be concluded that nailfold capillary changes in individual patients with systemic sclerosis are not useful in predicting organ involvement.

Adult↗
PubMed1994

Serum concentration of procollagen type I carboxyterminal propeptide in systemic sclerosis.

The serum level of procollagen type I carboxyterminal propeptide (P1CP), which has been used as an index of collagen synthesis in patients with various fibrotic diseases during the active stage, was measured using enzyme-linked immunosorbent assay in 61 patients with systemic sclerosis (SSc) and in 21 control subjects. The mean P1CP level in the SSc patients was significantly higher than in the normal controls (mean +/- SD, 326 +/- 319 vs 128 +/- 87 ng/ml; p < 0.005). In 36% of the SSc patients, the serum P1CP level was significantly elevated more than two standard deviations above the mean control value. The mean serum P1CP level in patients with diffuse SSc was significantly higher than in those with limited SSc (411 +/- 373 vs 255 +/- 199 ng/ml; p < 0.05). In addition, the SSc patients with elevated serum P1CP levels showed a significantly greater incidence of lung fibrosis and joint involvement than those with normal P1CP levels (p < 0.005 and p < 0.05, respectively). These results suggest that the serum P1CP level is a useful indicator of the severity of disease in SSc patients.

Adolescent↗
PubMed2004

[Nuclear auto-antibodies: a useful tool for the diagnosis, the classification and the prognosis of systemic sclerosis].

PURPOSE: Systemic sclerosis (SSc) like other connective tissue diseases is characterized by the occurrence of antinuclear auto-antibodies (ANA). We reviewed the diagnostic value and prognostic value of ANA in SSc. CURRENT KNOWLEDGE AND KEY POINTS: LeRoy proposed new criteria of limited forms of SSc which take into account the immunological profile of the patients. Anticentromere, antitopoisomerase I, antifibrillarin, anti-PM-Scl, antifibrillin or anti-RNA-polymerase I or III are considered as specific anti-autobodies. Whereas anticentromere antibodies are more associated with limited SSc and antitopoisomerase I with diffuse SSc, other auto-antibodies were associated with other organ involvement : anti-RNA-polymerase I or III and renal crisis, anti-U1-RNP and pulmonary hypertension and pulmonary fibrosis, etc. Patient with persistent antitopoisomerase I antibodies have a poor survival, those with antitopoisomerase antibodies that disappear with time have a better prognosis. FUTURE PROSPECTS AND PROJECTS: A better identification of the patients with SSc, an individual biological profile and organ involvement may help better management of the disease and better access to clinical trials. The development of new immunological tests and their clinical application should help us in that way.

Antibodies, Antinuclear↗
PubMed1995

Bronchial hyperreactivity in systemic sclerosis patients: influence of associated Sjögren's syndrome.

OBJECTIVE: To determine the frequency and relative risk of bronchial hyperreactivity to methacholine in systemic sclerosis patients with or without associated Sjögren's syndrome. METHODS: A prospective study of 56 patients with systemic sclerosis (42 with the diffuse and 14 with the limited variant; 24 with associated Sjögren's syndrome), 57 with primary Sjögren's syndrome, and 61 healthy controls. RESULTS: Bronchial hyperreactivity (BH) was present in 6.5% of the healthy controls, 25% of the systemic sclerosis patients without associated Sjögren's syndrome, 42.2% of those with primary Sjögren's syndrome, and in 50% of those with systemic sclerosis with associated Sjögren's syndrome. The presence of BH did not correlate with age, disease duration, chest radiograph abnormalities, respiratory, and immunological data. The subgroup of subjects with the limited variant of systemic sclerosis more frequently had associated BH than did those with the diffuse variant of the disease; coexisting Sjögren's syndrome further increased this frequency. CONCLUSIONS: In agreement with previous studies, we have confirmed the high prevalence of bronchial hyperreactivity in primary Sjögren's syndrome; systemic sclerosis likewise appears to be associated with an increased frequency of bronchial hyperreactivity compared with healthy control subjects. There is evidence also that the coexistence of Sjögren's syndrome and systemic sclerosis further increases the frequency and the calculated relative risk of developing bronchial hyperreactivity.

Bronchi↗
PubMed2001

Treatment of Raynaud's phenomenon with intravenous prostaglandin E1alpha-cyclodextrin improves endothelial cell injury in systemic sclerosis.

OBJECTIVE: To evaluate the efficacy and safety of prostaglandin (PG) E1alpha-cyclodextrin for Raynaud's phenomenon (RP) secondary to systemic sclerosis (SSc) and its effect on variables of immune activation and endothelial injury in SSc such as tumor necrosis factor-alpha (TNF-alpha), soluble interleukin 2 receptor (sIL-2R), circulating intercellular adhesion molecule-1 (cICAM-1), von Willebrand factor (vWF), and tissue-type plasminogen activator (t-PA). METHODS: We studied 36 women with SSc, 24 of them given three 60 microg intravenous PGE1alpha-cyclodextrin infusions on 5 consecutive days at 6 week intervals during the winter. RP symptoms and healing of digital lesions were evaluated. Twenty age matched healthy women were the controls. TNF-alpha, sIL-2R, cICAM-1, vWF, and t-PA were measured after the first and last infusion of PGEE1alpha-cyclodextrin and correlated with clinical features. RESULTS: RP symptoms improved in 87% of the patients. The benefit of each 5 day cycle lasted 4 or more weeks in 75%. PGE1alpha-cyclodextrin reduced the daily frequency of RP symptoms by 20% (p < 0.05), 41% (p < 0.005), and 53% (p < 0.0005) from baseline after the 1st, 2nd, and 3rd infusions, respectively. The severity of the attacks was reduced to a limited degree. In 12 of the 14 patients with digital lesions, these healed completely. Ten patients had mild side effects during treatment (headache, increased intestinal motility, flushing). TNF-alpha, sIL-2R, cICAM-1, vWF, and t-PA plasma concentrations were significantly higher in patients with SSc than controls (p < 0.05, p < 0.001). TNF-alpha, sIL-2R, and cICAM-1 were higher in diffuse SSc and patients with lung involvement. The plasma levels of cICAM-1 and t-PA were significantly reduced after the 1st infusion of PGE1alpha-cyclodextrin (both p < 0.005) and further reduced after the last (p < 0.0005 and p < 0.005). CONCLUSION: PGE1alpha-cyclodextrin reduces RP symptoms and plasma levels of the markers of endothelial injury in SSc, suggesting that an improvement of endothelial dysfunction contributes to its prolonged therapeutic effect.

Adult↗
PubMed1980

[Paralysis of digestive tract with lesions of myenteric plexuses. A new paraneoplastic syndrome (author's transl)].

A 67 years old man was admitted on July 1979 for nausea, dysphagia and rectal pain. At age 64 he had undergone radiotherapy on the lower lip for an epidermoid carcinoma. He remained then healthy. His medical history was negative with the exception of chronic bronchitis. He had never been exposed to toxic agents or drugs and had never left Europe. A few days after admission he suffered acute intestinal obstruction but at laparostomy no etiology was found. At the same time the patient complained of pain in all four limbs and he was found to have diffuse wasting of muscles, areflexia and distal sensory loss. No sign of dysautonomia was present. Physical examination was negative with the exception of a cervical lymphadenopathy. The lymph node biopsy showed an undifferentiated metastatic carcinoma. Negative investigations included: blood cells count; serum ionogram and immunoelectrophoresis; thyroid function tests; serological test for Chagas' disease. The following abnormalities were found: ESR: 55-105; CSF protein: 145 mg/100 ml and 1 cell mm3; whole blood folic acid: 1,7 mg/ml; Hbs antigen was present in blood; EMG showed evidence of denervation but motor conduction velocities were normal. By September the patient's weakness had increased and complete intestinal obstruction persisted. At oesophageal, gastric and duodenal fibroscopy no contraction was visible, and biopsies were negative. The patient died of peritonitis on October 5th, 1979. At necropsy peritonitis secondary to multiple perforation of the large bowel was found. No recurrence of the lip carcinoma or metastase or evidence of a primary carcinoma was found. Light microscopy showed no evidence of amyloidosis or scleroderma. Examination of the alimentary tract showed abnormalities restricted to the myenteric plexuses which varied from one level to another. In the small bowel there was hyperplasia of the smooth muscle and the myenteric plexuses were enlarged by marked proliferation of Schwann cells. Severe neuronal loss and nodules of Nageotte were also noted. Schwann cells proliferation was less marked in the stomach and large bowel. Lympho-histiocytic infiltration strictly confined to the region of the myenteric plexuses was present in oesophagus, stomach, large bowel and rectum. Mild chronic inflammatory lesions were also found in anterior and posterior spinal roots and semi-lunar ganglia. The striking feature of this case is the association of an undifferentiated carcinoma and a polyradiculoneuritis with a complete alimentary tract palsy of rapid onset, secondary to lesions restricted to the myenteric plexuses. The low folate level was insufficient to explain the neuropathy. Investigations showed no evidence of the usual causes of intestinal pseudo-obstruction: muscular, dysautonomic, toxic, plexic (idiopathic, familial, inflammatory), Chagas' disease). The clinical course, the pathological pictures of the alimentary tract and spinal roots and the association with a carcinoma suggest that our case may represent a paraneoplastic syndrome...

Aged↗
PubMed1995

Reduced bone mass and normal calcium metabolism in systemic sclerosis with and without calcinosis.

Forty-three female patients with systemic sclerosis divided into subgroups based on the extent of skin involvement and the presence of calcinosis, and 50 sex and age-matched healthy controls were investigated for bone mineral density (BMD) on the basis of radial (dual photon absorptiometry, Osteograph, NIM), lumbar, and total body measurements (dual energy X-ray absorptiometry, Lunar DPX, Lunar Corp.), and for parameters of calcium metabolism. The patients showed a lower BMD (mean +/- SD; mg/cm2) than the controls at the radial (313 +/- 69 vs 347 +/- 73; p < 0.005), lumbar (974 +/- 143 vs 1081 +/- 154; p < 0.005), and total body (997 +/- 82 vs 1075 +/- 109; p < 0.05) determinations. The patients with the diffuse form of skin involvement had lower values than those with the limited form. There was a negative correlation between BMD and the duration of the disease. The presence of calcinosis was not found to have any effect on BMD. Calcium metabolism was found to be normal in each subgroup. It may be concluded that generalized osteoporosis is a feature of systemic sclerosis, with and without calcinosis. The extent and duration of the disease may play a role in determining bone loss.

Absorptiometry, Photon↗